Constitutive upregulations of titin-based signalling proteins in KY deficient muscles

Neuromuscul Disord. 2006 Jul;16(7):437-45. doi: 10.1016/j.nmd.2006.05.013. Epub 2006 Jun 30.

Abstract

An increase in the expression of stretch/stress response elements in fast and slow muscles has been previously described in a transcriptional profiling of KY deficient muscles. Here, we have characterized the induction of this titin-based family of signalling proteins in ky/ky muscles at the protein level. Changes in expression of MLP, MARP2 and Xin have been related to the onset of dystrophic and adaptive changes that operate in ky/ky muscles. Our results indicate that induction of this set of genes is an early consequence of the interference caused by the absence of the KY protein. A search of muscle profiles of mouse models revealed such molecular hallmark only in muscles subjected to a single bout of eccentric contractions and specific titin mutants. Based on the role of this family as titin-based stress response molecules, it is suggested that titin structural/signalling instability is common to ky and titin mouse mutants and eccentric contractions.

MeSH terms

  • Adaptation, Physiological / physiology
  • Animals
  • Connectin
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism
  • LIM Domain Proteins
  • Mice
  • Mice, Mutant Strains
  • Muscle Proteins / chemistry
  • Muscle Proteins / genetics*
  • Muscle Proteins / metabolism*
  • Muscle, Skeletal / metabolism
  • Muscular Dystrophy, Animal / genetics*
  • Muscular Dystrophy, Animal / metabolism*
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism
  • Oligonucleotide Array Sequence Analysis
  • Peptide Hydrolases
  • Protein Kinases / chemistry
  • Protein Kinases / genetics
  • Protein Kinases / metabolism
  • Protein Structure, Tertiary
  • Signal Transduction / physiology*
  • Transcription, Genetic / physiology
  • Up-Regulation

Substances

  • Connectin
  • DNA-Binding Proteins
  • LIM Domain Proteins
  • Muscle Proteins
  • Nuclear Proteins
  • Xin protein, mouse
  • cysteine and glycine-rich protein 3
  • Protein Kinases
  • KY protein, mouse
  • Peptide Hydrolases